A Case of Tuberous Sclerosis with Spontaneous Rupture of Renal Angiomyolipoma / 대한신장학회잡지

Tuberous sclerosis is an autosomal dominant disorder characterized by seizures, mental retardation and hamartomatous tumors in multiple organs, including subependymal giant cell astrocytomas, cortical tubers, cardiac rhabdomyoma, pulmonary lymphangiomyomatosis and renal angiomyolipomas. However, these clinical features are not always present. The following is a report concerning a 30-year-old woman with tuberous sclerosis who suffered a spontaneous hemorrhage into the angiomyolipoma of the kidney and underwent curative selective renal embolization. The patient had a medical history of focal seizures. Moreover, brain magnetic resonance imaging revealed typical signs of tuberous sclerosis, and a high resolution computerized tomography of the chest showed bilateral nodules with irregular margins and multiple thin-walled cystic lesions. There was no family history of renal disease or of tuberous sclerosis. As such, this case exhibited rare clinical manifestations of tuberous sclerosis involving multiple organs.

Effects of Pretreatment with a Small Dose of Protamine on Protamine-induced Systemic Hypotension in the Heparinized Dog / 대한마취과학회지

INTRODUCTION: Protamine reversal of heparin anticoagulation in patients often produces profound hypotension. The present study was aimed to investigate the effectiveness of pretreatment with a small dose (0.5 mg.kg (-1)) of protamine in attenuating protamine-induced systemic hypotension in heparinized dogs. METHODS: Forty mongrel dogs were pretreated with either intravenous saline (control group, n=19) or protamine 0.5 mg.kg (-1) (protamine group, n=11) 5 minutes after heparin (300 IU.kg (-1), iv) during 1.5% halothane anesthesia. All dogs were then given protamine 3 mg.kg (-1) over a period of 30 s through right atrium 10 minutes after the pretreatment. Mean arterial pressure (MAP), mean pulmonary arterial pressure (MPAP), left ventricular end-diastolic pressure (LVEDP), heart rate (HR), and cardiac output and left circumflex coronary flow (LCX flow) via Doppler flowmeter were continuously recorded in baseline conditions and up to 20 minutes. Calculated parameters included cardiac index (CI), systemic and pulmonary vascular resistance indices (SVRI and PVRI), and LV dp/dt. RESULTS: In the control group, protamine infusion caused immediate but transient decreases of MAP (-42%), CI (-58%), LVEDP (-53%) and dP/dt (-25%), and increases of MPAP (39%), SVRI (38%) and PVRI (4.0 fold). Protamine pretreatment significantly attenuated but not completely blocked the hemodynamic responses to protamine. LCX flow increased significantly (213~258%) immediately after protamine infusion in both groups. CONCLUSIONS: These results suggest that, in the heparinized dog, pretreatment with a small dose of protamine attenuates the degree of hypotension that may follow the rapid administration of protamine.

Effects of Acute Myocardial Ischemia on Regional Function of the Remote Nonischemic Myocardium in Dogs / 대한마취과학회지

INTRODUCTION: During an acute myocardial ischemia, maintenance of overall ventricular function may depend on remote nonischemic myocardium. Whereas fentanyl has minimal hemodynamic effects, volatile anesthetics, including halothane and isoflurane cause negative inotropic and lusitropic effects in normal myocardium. This investigation examined the effects of volatile anesthetics in comparision with fentanyl on compensatory responses to brief left anterior descending coronary artery (LAD) occlusion in remote normal myocardium (left circumflex coronary artery (LCX) supply) in an open-chest canine model. METHODS: Thirty-six mongrel dogs, acutely instrumented for measurement of pressure (left ventricle (LV) and aorta), flows (pulmonary trunk and LCX) and dimensions in ischemic and non-ischemic myocardium, were subjected to a 10-min LAD occlusion during fentanyl (n=10), halothane (n=13), or isoflurane (n=13) anesthesia. Regional contractile function was assessed using percent systolic shortening (%SS) and the preload recruitable stroke work slope (Mw). Diastolic function was evaluated using a regional time constant for intramyocardial pressure decline of LV (IMPtau), peak lengthening rate (dL/dtmax) and a regional chamber stiffness constant (Kp). RESULTS: Acute LAD occlusion caused immediate deterioration of anterior wall function similarly without changes in cardiac index, mean arterial pressure and dP/dtmax in all three groups. LV end-diastolic pressure (LVEDP), LVPtau, and heart rate increased and dP/dtmin decreased to the same extent with regional myocardial ischemia in all groups. During fentanyl anesthesia, acute myocardial ischemia was associated with an increase in %SS (26%) and Mw (48%) in LCX area without changes in IMPtau and dL/dtmax. With halothane or isoflurane anesthesia, %SS, Mw and IMPtau showed similar changes as those in fentanyl in response to LAD occlusion. However, dL/dtmax was increased (47 and 45% in the halothane and isoflurane groups, respectively) and Kp was increased (34 and 33% in the halothane and isoflurane groups, respectively) less compared to fentanyl (78%). Enhanced function in LCX zone was associated with a comparable increase (21~28% from baseline) in LCX flow in all groups. CONCLUSION: Enhanced regional contractility following acute coronary occlusion in nonischemic myocardium during fentanyl anesthesia is well-preserved with volatile anesthetics in an open-chest canine model. In addition, diastolic functions are also enhanced rather than depressed during anesthesia with volatile anesthetics. Halothane and isoflurane, however, do not differ in the compensatory responses to acute regional ischemia.

A Case of Follicular Carcinoma Associated with Hashimoto's Thyroiditis Arising in a Thyroglossal Duct Remnant / 대한내분비학회지

Thyroglossal duct remnants are common congenital abnormalities of the thyroid development but malignant tumors arising in the thyroglossal duct remnants are rare, occuring in less than 1%. In the literature, about 160 cases of malignancy in the thyroglossal duct remnant have been reported. Among the various types of carcinoma being found from the thyroglossal duct remnants, most are papillary carcinoma(79.8%) and Mixed follicular-papillary carcinoma(9.5%). Others are squamous cell carcinoma(7.6%), follicular carcinoma(1.2%), and anaplastic carcinoma(0.6%). Hashimotos thyroiditis is a common autoimmune disease of the thyroid gland. Malignant tumors of the thyroid gland with Hashimotos thyroiditis are rare disorder except malignant lymphoma whose association with Hashimotos thyroiditis is well known. Malignant tumors associated with Hashimotos thyroiditis arising in a thyroglossal duct remnant are extremely rare. Recently the authors experienced a case of follicular carcinoma associated with Hashimotos thyroiditis arising in a thyroglossal duct remnant, which was removed by local excision and reported with literature review.

Evaluation of Left Ventricular Systolic Function by-M-Mode Echo/Phonocardiography and Automated Border Detection(ABD) Echocardiography

BACKGROUND: The rate of rise of left ventricular(LV) systolic pressure, dp/kt, and the peak ejection rate(PER) of LV may be more accurate indices for assessing LV systolic function than ejection fraction(EF). Both can be easily obtained by noninvasive methods, M-mode echo/phonocardiography and automated border detection(ABD) echocardiography, respectively. Mean dp/dt by M-mode echo/phonocardiography and PER by ABD echocardiography were compared with mean dp/dt by Doppler echocardiography. METHODS: Twenty-one adult patients who had normal sinus rhythm, mitral regurgitant signals by continuous wave Doppler echocardiography and an apical 4 chamber view of LV in which at least 75% of the endocardium was clearly visualized were selected for our study. The off-line estimation of LV end-diastolic volume (1) were performed by the method of disc, after manually tracing the endocardial border on the apical 4 chamber view and EF was calculated. M-mode echocardiography of the aortive valve and phonocardiography were simultaneously recorded on the strip chart to measure the isovolumic contraction time(dtM). A blood pressure (2) and LV end-diasolic pressure(LVEDP) was assigned 20mmHg in patients with Q wave myocardial infarction or EF< or =40% and 10mmHg in others. The mean dp/dtM during isovolumic contraction time was calculated as (aortic diastolic pressure-LVEDP)/dtM and was compared with the Doppler-derived mean rate of LV pressure rise(dp/dtDoppler) over the time period between velocities of 1 and 3m/sec on the ascending slope of the Doppler velocity spectrum. ABD system was used to measure the changes in LV volume and PER on the apical four chamber view. PER was compared with mean dp/dtDoppler. RESULTS: Mean dp/dtM positively correlated with mean dp/dtDoppler(r=0.73, p<0.001), but the limits of agreement between two methods were somewhat wide(-659-937mmHg/sec). PER also positively correlated with dp/dtDoppler and EF(r=0.73, p<0.001 ; r=0.80, p<0.001). CONCLUSIONS: Mean dp/dtM by M-mode echo/phomocardiography and PER by ABD echocardiography may be useful indices for assessing LV systolic function.